Overview
Papillary renal cell carcinoma (also called papillary kidney cancer or PRCC) is a type of kidney cancer that starts in the cells lining the small tubes inside the kidney. It is the second most common type of kidney cancer, making up about 10 to 20 percent of all renal cell carcinomas. There are two subtypes: Type 1 and Type 2. Type 1 tends to grow more slowly and often has a better outlook, while Type 2 can be more aggressive. The disease affects the body by forming one or more tumors in the kidney. In early stages, many people have no symptoms at all. As the tumor grows, symptoms may include blood in the urine, pain in the side or lower back, a lump or mass in the abdomen, unexplained weight loss, fatigue, and fever. Some cases are found by accident during imaging tests done for other reasons. Most papillary renal cell carcinomas occur sporadically, meaning they happen without a family history. However, some cases are hereditary and linked to specific gene changes, particularly in the MET gene (Type 1) or the FH gene (hereditary leiomyomatosis and renal cell cancer syndrome, which can cause Type 2). Treatment typically involves surgery to remove part or all of the affected kidney. For advanced or metastatic disease, newer targeted therapies and immunotherapy drugs have become available, though treatment options are more limited compared to the more common clear cell type of kidney cancer. Clinical trials are actively exploring better treatments for this cancer.
Key symptoms:
Blood in the urine (may appear pink, red, or dark)Pain or aching in the side or lower backA lump or mass felt in the abdomen or sideUnexplained weight lossPersistent fatigue or tirednessFever that comes and goes without infectionLoss of appetiteNight sweatsAnemia (low red blood cell count)High blood pressureSwelling in the legs or anklesGeneral feeling of being unwell
Variable
Can be inherited in different ways depending on the underlying gene
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
3 eventsNational Cancer Institute (NCI) — PHASE2
Centre Leon Berard — PHASE2
AstraZeneca — PHASE3
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Papillary renal cell carcinoma.
3 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Papillary renal cell carcinoma.
Community
No community posts yet. Be the first to share your experience with Papillary renal cell carcinoma.
Start the conversation →Latest news about Papillary renal cell carcinoma
Disease timeline:
New recruiting trial: Axitinib +/- Pembrolizumab in First Line Treatment of mPRCC
A new clinical trial is recruiting patients for Papillary renal cell carcinoma
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What subtype of papillary renal cell carcinoma do I have (Type 1 or Type 2), and how does that affect my treatment and outlook?,What stage is my cancer, and has it spread beyond the kidney?,Should I have genetic testing to see if my cancer is hereditary, and should my family members be tested?,What are my treatment options, and what are the benefits and side effects of each?,Are there any clinical trials available for papillary renal cell carcinoma that I might be eligible for?,How often will I need follow-up imaging and blood tests after treatment?,What can I do to protect my remaining kidney function?
Common questions about Papillary renal cell carcinoma
What is Papillary renal cell carcinoma?
Papillary renal cell carcinoma (also called papillary kidney cancer or PRCC) is a type of kidney cancer that starts in the cells lining the small tubes inside the kidney. It is the second most common type of kidney cancer, making up about 10 to 20 percent of all renal cell carcinomas. There are two subtypes: Type 1 and Type 2. Type 1 tends to grow more slowly and often has a better outlook, while Type 2 can be more aggressive. The disease affects the body by forming one or more tumors in the kidney. In early stages, many people have no symptoms at all. As the tumor grows, symptoms may include
At what age does Papillary renal cell carcinoma typically begin?
Typical onset of Papillary renal cell carcinoma is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Papillary renal cell carcinoma?
Yes — 3 recruiting clinical trials are currently listed for Papillary renal cell carcinoma on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Papillary renal cell carcinoma?
21 specialists and care centers treating Papillary renal cell carcinoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.